28 cases of primary sclerosing cholangitis / 대한내과학회지

ABSTRACT

Backgroud Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic hepatobiliary disease of unknown etiology, a characterized by inflammation, scarring and obliteration of bile ducts, eventually leading to biliary cirrhosis and liver failure. In Korea, reports on PSC have only been done anecdotally and there are hardly any large scale studies. METHODS: We retrospectively analyzed the clinical, laboratory, radiologic and histologic feature of 28 patients, who were diagnosed in Asan Medical Center during the last decade. A diagnosis of PSC was made by typical direct cholangiographic findings. RESULTS: The median age of the enrolled patients at diagnosis was 39.3 years, and there was a preponderance of men (MF = 2.11). The major clinical features were abdominal pain and abnormal liver function tests of cholestatic pattern. The rate of positivity for autoantibody was 30.8% (8/26). On cholangiogram, 82.1% had evidence of both extra- and intrahepatic bile duct involvement, 10.7% of intrahepatic involvement only, and 7.1% of hilar involvement only. The pancreatic duct involvement was seen in 10.5% (2/19). The incidence of associated inflammatory bowel disease was 50% (6/12), UC in 5 cases and Crohn's disease in 1 case, respectively. Endoscopic or radiologic intervention for the dilatation of narrowed bile ducts were performed in 14.3% and 71.4%, respectively. Two cases (7.1%) underwent liver transplantation. CONCLUSION: Although PSC is a rare disease in Korea, we prospect that more cases will be reported as concern and knowledge of this entity increase.